Metallothionein in kidney and liver of the macular mouse as an animal model of Menkes' kinky hair disease.

N. Shiraishi, S. Kondoh, Y. Hiraki, K. Aono, Takehito Taguchi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

The tissue copper and metallothionein-Cu (MT-Cu) content of kidney and liver were measured in mutant (hemizygous macular male and homozygous macular female), heterozygous macular female and normal mouse. The tissue copper and MT-Cu contents in kidneys from 7-8 day mutants and heterozygotes were significantly greater than those of the normal kidney. Marked elevations in kidney copper and MT-Cu contents were also observed in the 8-9 week mutant (which achieved long-term survival with a single dose of subcutaneous copper administered at day 7) and in the heterozygote. The L-[35S]cystine incorporation experiments also revealed an abnormal synthesis of renal MT in the 8-9 week mutant and in the heterozygote. In contrast to kidney Cu levels, the tissue copper and MT-Cu contents of 7-8 day normal livers were extremely high, whereas the tissue copper and MT-Cu contents of mutant and heterozygote livers were extremely low. The tissue copper contents of livers of 8-9 week mutants and heterozygotes were slightly low compared to normal, and the MT-Cu contents of livers of the 8-9 week mouse were extremely low in all groups. In contrast to the changes in copper content, the changes in tissue zinc and MT-Zn contents in kidney and liver were slight in the 7-8 day and 8-9 week mouse.

Original languageEnglish
Pages (from-to)227-233
Number of pages7
JournalPhysiological Chemistry and Physics and Medical NMR
Volume19
Issue number4
Publication statusPublished - 1987

Fingerprint

Menkes Kinky Hair Syndrome
Metallothionein
Liver
Animals
Animal Models
Heterozygote
Tissue
Kidney
Copper
Cystine
Zinc
copper thionein

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Biochemistry
  • Biophysics
  • Physiology
  • Physiology (medical)
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Metallothionein in kidney and liver of the macular mouse as an animal model of Menkes' kinky hair disease. / Shiraishi, N.; Kondoh, S.; Hiraki, Y.; Aono, K.; Taguchi, Takehito.

In: Physiological Chemistry and Physics and Medical NMR, Vol. 19, No. 4, 1987, p. 227-233.

Research output: Contribution to journalArticle

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